Dermal Epstein Barr Virus–Associated Leiomyosarcoma: Tocsin of Acquired Immunodeficiency Syndrome in Two Children

Abstract

Although rare in childhood, a relatively high incidence of smooth muscle tumors are recognized in patients with AIDS, mainly in association with Epstein Barr virus (EBV) infection. Although EBV-associated smooth muscle tumors have been documented rarely in the subcutis of AIDS patients, dermal involvement has not been described to date. This report describes dermal EBV-associated leiomyosarcomas (EBV-LMS) with a nodular but superficial plaque-like appearance on the lower limbs of 2 males, 9 and 12 years old. Histopathological assessment of the excised lesions demonstrated hypercellular mitotically active dermal tumors with hyperchromatic spindle and round cells, arranged in short fascicles and sheets, with microfoci of necrosis. A smooth muscle immunophenotype, including prominent desmin immunopositivity, and positive EBV-encoded RNA in situ hybridization investigation confirmed a diagnosis of EBV-LMS. Subsequent HIV seropositivity and AIDS were confirmed in both patients. Both patients also had pulmonary tuberculosis and received antituberculous therapy. Patient 1 had a 3 cm re-excision of the prior tumor site. He received highly active antiretroviral therapy, completed 6 months of antituberculous therapy, achieved immune reconstitution and viral suppression and is tumor-free 2 years after tumor excision. Patient 2 died before further therapy. The immune status, presence, and appropriate therapy of co-existent systemic infection and highly active antiretroviral therapy in AIDS patients with EBV-LMS are crucial to a favorable outcome.

Extra-uterine myoid tumours in patients with acquired immunodeficiency syndrome: a clinicopathological reappraisal

“… Immunohistochemical scoring: −, negative; 0, 1–5%; 1+, 6–25% positivity; 2+, 26–50%, positivity; 3+, 51–75% positivity; 4+, >75% positivity; TS/G: total score and grading of LMS according to FNCLCC 19,23 ; I: intermediate‐grade (score: 4–5: grade 2); H: high‐grade (score, 6–8: grade 3); Pl: pleomorphism (score: 2, conventional LMS; 3, pleomorphic LMS); N: necrosis (0: no necrosis; 1: <50% necrosis); Mi: mitoses (1, <10/10HPF; 2: 10–19/10HPF; 3, >19/10HPF), (HPF, high power field; single HPF = 0.1734mm 2 ). …”

Perinatally acquired HIV infection in adolescents from sub-Saharan Africa: a review of emerging challenges

“…Cases of atypical and rare malignant diseases in HIV-infected adolescents have been reported. 44,46,47 …”

Sarcomas other than Kaposi sarcoma occurring in immunodeficiency

“…In both immunodeficient populations, more than half of the reported sarcomas were leiomyosarcoma. Fifty-four leiomyosarcomas occurred in solid organ transplant recipients [10, 14–57], and 47 occurred in people with HIV [58–86]. Thus, 57.8% of all sarcomas reported in immunodeficient hosts were leiomyosarcomas.…”